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非常年轻的杜氏肌营养不良症患者的心电图异常先于心脏功能障碍的发生。

Electrocardiographic abnormalities in very young Duchenne muscular dystrophy patients precede the onset of cardiac dysfunction.

机构信息

The Heart Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati School of Medicine, Cincinnati, OH, USA.

出版信息

Neuromuscul Disord. 2011 Jul;21(7):462-7. doi: 10.1016/j.nmd.2011.04.005. Epub 2011 May 14.

Abstract

Overt cardiac involvement in Duchenne muscular dystrophy (DMD) typically occurs later in the disease. The primary aim was to estimate the proportion of young (<6 years of age) DMD patients with manifestations of cardiac disease by electrocardiography (ECG). Secondary aims were to assess associations between ECG abnormalities and evidence of cardiac disease by echocardiography, as well as to estimate the relationship between dystrophin mutation site and an abnormal ECG. Seventy eight steroid-naive DMD patients <6 years of age were identified. ECG abnormalities were identified in 78%, with LV pathology being the most commonly identified pattern. Only one echocardiogram was abnormal. There was no statistically significant relationship identified between ECG abnormalities and dystrophin genotype. ECG abnormalities are common in very young DMD patients, signaling cardiac involvement well before the onset of clinical symptoms.

摘要

杜氏肌营养不良症(DMD)的心脏显性病变通常发生在疾病后期。本研究旨在通过心电图(ECG)评估年轻(<6 岁)DMD 患者中出现心脏疾病表现的比例。次要目标是评估心电图异常与超声心动图检查中心脏疾病证据之间的关系,并估计肌营养不良蛋白基因突变部位与异常心电图之间的关系。本研究共纳入 78 例未经激素治疗的<6 岁 DMD 患者。78%的患者存在心电图异常,左心室病变是最常见的异常模式。只有 1 例超声心动图异常。心电图异常与肌营养不良蛋白基因型之间无统计学显著相关性。结论:非常年轻的 DMD 患者中,心电图异常很常见,这表明在出现临床症状之前就已经有心脏受累。

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